WAGR SYNDROME

WAGR syndrome is a rare genetic condition that can affect both boys and girls. Babies born with WAGR syndrome often have eye problems, and are at high risk for developing certain types of cancer, and a range of developmental delays. The term “WAGR” stands for the first letters of the physical and mental problems associated with the condition:

• (W)ilms’ Tumor, the most common form of kidney cancer in children.
• (A)niridia, some or complete absence of the colored part of the eye, called the iris (singular), or irises/irides (plural).
• (G)enitourinary problems, such as testicles that are not descended or hypospadias (abnormal location of the opening for urination) in boys, or genital or urinary problems inside the body in girls.
• (R)ange of developmental delays.

Most people who have WAGR syndrome have two or more of these conditions. Also, people can have WAGR syndrome, but not have all of the above conditions.

Other names for WAGR syndrome that are used are:

• WAGR Complex
• Wilms’ Tumor-Aniridia-Genitourinary Anomalies-Mental Retardation Syndrome
• Wilms’ Tumor-Aniridia-Gonadoblastoma-Mental Retardation Syndrome
• Chromosome 11p deletion syndrome
• 11p deletion syndrome
  

The cause of WAGR syndrome is deletion of a group of genes located on chromosome number 11 (11p13 – the “p13” refers to the specific place on chromosome 11 that is affected). Chromosomes are packages of genetic characteristics. There are 22 pairs of chromosomes that are the same in males and females. The 23rd pair determines a person’s sex with males having an X and Y chromosome and females having two X chromosomes.








*Source: International WAGR Syndrome Association, WAGR.org